Behcet Disease

DEFINITION: Behcet’s Disease was first defined in 1937 by Prof. Dr. Hulusi Behçet as a disease causing recurring lesions inside the mouth and genital region and uveitis (intraocular inflammation).Behcet’s Disease is a chronic inflammatory disease which may occur in almost all systems of the body and may cause the involvement of the skin, joints, vessels, and organs of the respiratory system, central nervous system, and digestive system. Aching aphthae in the mouth and ulcers on the skin and genital region are commonly seen. Findings and symptoms of the disease are characterized by inflammations in the involved organs. However, although some symptoms continue to appear for a long time, there are some periods that change from one person to another when lesions are not present and there are no complaints.

Figure 1.  Severe anterior uveitis resulted in the formation of hypopyon within the anterior chamber (left) and posterior synechiae formation due to inadequate, delayed treatment (right).

RISK FACTORS AND CAUSES: Behcet’s Disease is an autoimmune disease (a response by the immune system against its own tissues of the body). It may result from genetic predisposition. It is more common in countries such as Turkey and Japan, while it is less common in North America. Ocular involvement is more common in men compared with women. Ocular involvement also indicates that the course of the disease will be severe. The most important indication that the course of the eye disease will be severe is common and intense inflammation symptoms called activation. Particularly, the medications must be used regularly and as recommended, otherwise, the disease may inflame.

FINDINGS: Eye involvement of Behcet’s Disease can be like anterior uveitis, posterior uveitis, or panuveitis where anterior and posterior uveitis are found together. During attacks, a decrease in vision occurs due to temporal damage in eye tissues. Patients experience redness and feel sensitive to light. Among these symptoms, there is another one called "hipopion" - a more common symptom found in patients with Behcet’s Disease. This symptom indicates that the disease has the risk of leaving severe damage. After recurrent and severe attacks, permanent loss of vision may occur due to damage in internal layers of the eye (retina). The most important finding of the attacks is the impairment in vision. Patients are recommended to be careful about early diagnosis of attacks and to immediately consult their ophthalmologist even on suspicion of attack development in addition to regular check-up examinations.

DIAGNOSTIC TESTS: Diagnosis is made from the medical history of the patient and findings detected during examination. Along with typical intraoral and genital region ulcers, skin symptoms indicate existence of the disease. Detecting findings of uveitis in biomicroscopic eye examination is significant for diagnosis. Sometimes such supplementary investigations as Fundus Fluorescein Angiography, Optical Coherence Tomography and visual field are required to evaluate the involvement of the optic nerve and retina.

TREATMENT: Since Behcet’s Disease affects other organs along with the eye, adopting a multi-disciplinary approach is significant in treatment and follow-up. Therefore, the patient should be followed by dermatology, rheumatology, and neurology. Patients with anterior uveitis are given drops and creams with corticosteroid to suppress the inflammation. Drops that can enlarge the pupil are administered in order to separate the cohesion between the iris and eye lens. Depending on the status of the disease, local injection treatments around the eye can be applied. Systemic corticosteroid and medications suppressing the immune system can be administered in certain doses, singly or combined, depending on the severity of the disease, to patients with posterior uveitis or panuveitis. It is quite important for patients to attend follow-up examinations regularly to monitor the progress of the disease, to determine the type, dosage and adverse effects of the medical treatment, and to detect complications of the disease. Such eye diseases as cataract and glaucoma that may develop over time should be treated.

Hipopion is the white area formed because of accumulation of leucocytes in the anterior chamber angle due to gravity as a result of common cell reaction developing in the front of the eye (anterior chamber).